23 to 25 May 2022 Case Hemophagocytic Lymphohistiocytosis HLH associated with liver injury in Sarcoidosis hepatologist on the horns of a dilemma Dr Prajna Anirvan Department of Gastroenterology ID: 1032323
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1. Preceptorship Programme23 to 25 May 2022Case: Hemophagocytic Lymphohistiocytosis (HLH) associated with liver injury in Sarcoidosis – hepatologist on the horns of a dilemmaDr Prajna AnirvanDepartment of GastroenterologySCB Medical College, CuttackIndia.
2. Background History24-year old unmarried male, mason by occupation, diagnosed with cutaneous sarcoidosis 9 months ago, on maintenance steroids in remission:No history of diabetes, alcohol intake, or IV drug abuseJaundiceFever Referral due to non-improvement
3. Index PresentationJaundice x 1 monthNo history suggestive of cholestasis/nausea/vomiting/alternative medicine intake. Dragging sensation in right upper abdomenFever x 2 monthsHigh grade continuous fever without chill and rigor, subsided on taking antipyreticsPhysical examination: BMI 23 kg/m², Icterus, Temperature: 102°FAbdomen: Liver 18 cm, firm, non-tender, spleen enlarged 2 cm below LCM, no free fluid
4. Initial Labs and Provisional DiagnosisParameterValueHemoglobin (gm/dL)11ESR (mm AEFH)/CRP (mg/L)638Total leukocyte Count (x10³/mm³)2.8Platelet Count (x10³/mm³)210Total Bilirubin/Direct Bilirubin (mg/dL)7.2/4.1AST (IU/L)727ALT (IU/L)606ALP (IU/L)817Albumin/globulin (gm/dL)3.1/3.0INR1.29Creatinine (mg/dL)0.7Na/K (mmol/L)128/4.3Fasting Blood Sugar (mg/dL)89USG abdomen – Hepatomegaly (21 cm) with Grade 2 Fatty Changes, IHBR normal, no SOL, Splenomegaly, Few mildly enlarged porta hepatic nodes (13 mm), Portal vein - not dilated.MRI abdomen with MRCP – Hepatomegaly, no SOL, IHBR normalSplenomegalyUGI endoscopy: Normal mucosal study till D2, no varicesEtiological work up – HAV/HEV IgM: non reactive HbsAg, HCV, Anti-HBc Total - negativeCMV, EBV, HIV, COVID-19 RTPCR‐ Negative Malaria/Dengue: Neg Blood, Urine Cultures : Sterile Procalcitonin: 0.2 μg/LANA- negative, AIH profile- negativeMantoux Test- Non reactive, IGRA - negative? Sarcoidosis with hepatic involvement not responding to steroids
5. Clinical Course and Further Work upHigh spiking feverJaundice Oral steroids increased to 40 mg/day, antibiotics started, not responding.Liver biopsy and bone marrow biopsy done.ParameterValueReference rangeSerum triglyceride (mg/dL)146345-150Serum ferritin (μg/L)>200013-400Serum LDH (IU/L)1811 200-450Serum fibrinogen (mg/dL)73.6110-420Is itSecondary Hemophagocytic Lymphohistiocytosis(HLH)/ Macrophage Activation Syndrome(MAS)???In view of raised CRP and normal ESR
6. Liver Histopathology and Bone Marrow Study
7. ManagementIV Methylprednisolone 1 gm/day pulse dose started for treatment of HLH.Patient afebrile at Day 3.Oral cyclosporine at 2 mg/kg started.Day 7ParameterValueBilirubin (mg/dL)7.1AST (IU/L)267ALT (IU/L)231ALP (IU/L)750Day 30Bilirubin (mg/dL)3.9AST (IU/L)76ALT (IU/L)81ALP (IU/L)510ParameterValueBilirubin(mg/dL)0.6Day 90AST(IU/L)67ALT53ALP245USG(abdomen)- Grade II fatty liver, liver span-15 cm
8. QuestionsHow commonly is sarcoidosis complicated by HLH/MAS?Was hepatosplenomegaly due to HLH per se or due to sarcoidosis involving the liver?How to distinguish hepatic sarcoidosis from HLH in the background of deranged LFT?Did low dose steroid (10 mg/day) which patient was taking suppress HLH from manifesting initially?